ELECTRONIC LETTER

PRKAR1A, one of the Carney complex genes, and its locus (17q22-24) are rarely altered in pituitary tumours outside the Carney complex

F Sandrini^1,*, L S Kirschner^1,*, T Bei¹, C Farmakidis¹, J Yasufuku-Takano², K Takano², T R Prezant³, S J Marx⁴, W E Farrell⁵, R N Clayton⁵, L Groussin⁶, J Bertherat⁶, C A Stratakis¹

¹ Unit on Genetics and Endocrinology (UGEN), Developmental Endocrinology Branch (DEB), National Institute of Child Health and Human Development (NICHD), Bethesda, Maryland, USA
² Department of Nephrology and Endocrinology, University of Tokyo School of Medicine, Tokyo 113-8655, Japan
³ University Children's Genetics Laboratory and Children's Hospital, Los Angeles, CA 90027, USA
⁴ Metabolic Diseases Branch, National Institute of Diabetes, Digestive and Kidney Diseases (NIDDK), National Institutes of Health (NIH), Bethesda, MD 20892, USA
⁵ North Staffordshire Hospital, Keele University, Stoke on Trent, Staffordshire ST4 7QB, UK
⁶ Département d'Endocrionlogie, Institut Cochin, 75014 Paris, France

Correspondence to:
Correspondence to:
Dr C A Stratakis, Unit on Genetics and Endocrinology, DEB, NICHD, NIH, Building 10, Room 10N262, 10 Center Drive MSC1862, Bethesda, Maryland 20892-1862, USA;
stratakc@cc1.nichd.nih.gov

Keywords: protein kinase A; pituitary tumours; chromosome 17; tumour suppressor gene

The first 150 words of the full text of this article appear below.

The tumour suppressor gene encoding the cAMP dependent protein kinase A (PKA) type I- regulatory subunit (RI), PRKAR1A, has been mapped to chromosome 17q22-24 and is often mutated in the Carney complex (CNC),^1,2 a multiple neoplasia and lentiginosis syndrome inherited in an autosomal dominant manner.^3,4 The complex was first described as an association of lentigines, primary pigmented nodular adrenocortical disease (PPNAD), and a variety of endocrine and non-endocrine tumours (cardiac and breast myxomas).^5–7 Growth hormone (GH) and prolactin (PRL) secretion abnormalities have been found in over two-thirds of patients with CNC^8,9; in some cases, pituitary somatomammotrophic hyperplasia was also seen.⁸ GH producing adenomas (which also secrete small amounts of PRL) have been reported with increased frequency in patients with CNC; it was suggested that tumours in these patients develop in situ from precursor benign hyperplasia, following a sequence of genetic events not unlike the one described in . . . [Full text of this article]

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